人 RNH1 抗体对
01AB02270
Ribonuclease/angiogenin inhibitor 1 (RNH1) is a cytosolic leucine-rich repeat (LRR) protein that serves as a potent inhibitor of pancreatic-type ribonucleases, including RNase 1, 2, 4, and angiogenin. It forms a horseshoe-shaped structure that binds these enzymes with femtomolar affinity, protecting intracellular RNA from degradation and inhibiting pro-angiogenic activities. Beyond its role in RNA stability, RNH1 associates with ribosomes to regulate mRNA-specific translation, notably promoting the translation of the erythroid transcription factor GATA1 during erythropoiesis. Clinically, biallelic loss-of-function variants in RNH1 cause a severe congenital disorder characterized by neurodevelopmental delay, seizures, myopathy, and cataracts. It is also implicated in hematopoiesis, inflammatory control, and oxidative stress defense.
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技术参数
- 靶点基因名
- RNH1
- 基因别名
- PRI; RNH
- 蛋白别名
- ribonuclease/angiogenin inhibitor 1; RNH1
- UniProt
- P13489
- 物种
- Human
产品详情
- 靶点名称
- RNH1
- 蛋白名称
- ribonuclease/angiogenin inhibitor 1
- 蛋白别名
- Ribonuclease inhibitor; Placental ribonuclease inhibitor; Placental RNase inhibitor; Ribonuclease/angiogenin inhibitor 1; RAI
- 靶点基因名
- RNH1
- 基因别名
- PRI; RNH
- UniProt
- P13489
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Ribonuclease/angiogenin inhibitor 1 (RNH1) is a cytosolic leucine-rich repeat (LRR) protein that serves as a potent inhibitor of pancreatic-type ribonucleases, including RNase 1, 2, 4, and angiogenin. It forms a horseshoe-shaped structure that binds these enzymes with femtomolar affinity, protecting intracellular RNA from degradation and inhibiting pro-angiogenic activities. Beyond its role in RNA stability, RNH1 associates with ribosomes to regulate mRNA-specific translation, notably promoting the translation of the erythroid transcription factor GATA1 during erythropoiesis. Clinically, biallelic loss-of-function variants in RNH1 cause a severe congenital disorder characterized by neurodevelopmental delay, seizures, myopathy, and cataracts. It is also implicated in hematopoiesis, inflammatory control, and oxidative stress defense.